Mouse Basement membrane-specific heparan sulfate proteoglycan core protein ELISA Kit | Hspg2 elisa kit

Mouse Basement membrane-specific heparan sulfate proteoglycan core protein ELISA Kit

Cat. Num. AAA764771

• Gene Names: HSPG2; PLC; SJA; SJS; HSPG; SJS1; PRCAN
• Reactivity: Mouse
• Synonyms: Basement membrane-specific heparan sulfate proteoglycan core protein; Mouse Basement membrane-specific heparan sulfate proteoglycan core protein ELISA Kit; Hspg2/PLC/HSPG/PRCAN/SJA/SJS/SJS1/Perlecan/endorepellin (domain V region)/heparan sulfate proteoglycan 2/perlecan proteoglycan/PLCSchwartz-Jampel syndrome 1 (chondrodystrophic myotonia; Hspg2 elisa kit

• Reactivity: Mouse
• Sequence Length: 4391

• Samples: Serum, plasma and other biological fluids.
• Assay Type: Sandwich ELISA, Double Antibody
• Detection Range: 0.156-10ng/ml
• Sensitivity: 0.094ng/ml
• Preparation and Storage: Store at 4 degree C if kit is to be used within 1 week. Stable for 6 months (if micro ELISA Plate, Lyophilized Standard and Concentrated Biotinylated Detection Protein stored at-20 degree C. Other components at 2-8 degree C). Stable for 12 months (if the entire kit is stored at-20 degree C).

Typical Testing Data

Typical Standard Curve

Related Product Information for Hspg2 elisa kit

Principle of the Assay||This kit was based on sandwich enzyme-linked immune-sorbent assay technology. anti-Mouse Hspg2 antibody was pre-coated onto 96-well plates. And the biotin conjugated anti-Mouse Hspg2 antibody was used as detection antibodies. The standards, test samples and biotin conjugated detection antibody were added to the wells subsequently, and wash with wash buffer. HRP-Streptavidin was added and unbound conjugates were washed away with wash buffer. TMB substrates were used to visualize HRP enzymatic reaction. TMB was catalyzed by HRP to produce a blue color product that changed into yellow after adding acidic stop solution. The density of yellow is proportional to the Mouse Hspg2 amount of sample captured in plate. Read the O.D. absorbance at 450nm in a microplate reader, and then the concentration of Mouse Hspg2 can be calculated.

NCBI and Uniprot Product Information

• NCBI GI #: 126012571
• NCBI GeneID: 3339
• NCBI Accession #: NP_005520.4
• NCBI GenBank Nucleotide #: NM_005529.6
• Molecular Weight: 468,830 Da
• NCBI Official Full Name: basement membrane-specific heparan sulfate proteoglycan core protein isoform b
• NCBI Official Synonym Full Names: heparan sulfate proteoglycan 2
• NCBI Official Symbol: HSPG2
• NCBI Official Synonym Symbols: PLC; SJA; SJS; HSPG; SJS1; PRCAN
• NCBI Protein Information: basement membrane-specific heparan sulfate proteoglycan core protein
• UniProt Protein Name: Basement membrane-specific heparan sulfate proteoglycan core protein
• Protein Family: Basement membrane-specific heparan sulfate proteoglycan core protein
• UniProt Gene Name: HSPG2
• UniProt Synonym Gene Names: HSPG; PLC
• UniProt Entry Name: PGBM_HUMAN

NCBI Description

This gene encodes the perlecan protein, which consists of a core protein to which three long chains of glycosaminoglycans (heparan sulfate or chondroitin sulfate) are attached. The perlecan protein is a large multidomain proteoglycan that binds to and cross-links many extracellular matrix components and cell-surface molecules. It has been shown that this protein interacts with laminin, prolargin, collagen type IV, FGFBP1, FBLN2, FGF7 and transthyretin, etc., and it plays essential roles in multiple biological activities. Perlecan is a key component of the vascular extracellular matrix, where it helps to maintain the endothelial barrier function. It is a potent inhibitor of smooth muscle cell proliferation and is thus thought to help maintain vascular homeostasis. It can also promote growth factor (e.g., FGF2) activity and thus stimulate endothelial growth and re-generation. It is a major component of basement membranes, where it is involved in the stabilization of other molecules as well as being involved with glomerular permeability to macromolecules and cell adhesion. Mutations in this gene cause Schwartz-Jampel syndrome type 1, Silverman-Handmaker type of dyssegmental dysplasia, and tardive dyskinesia. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, May 2014]

Uniprot Description

HSPG2: Integral component of basement membranes. Component of the glomerular basement membrane (GBM), responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. It serves as an attachment substrate for cells. Plays essential roles in vascularization. Critical for normal heart development and for regulating the vascular response to injury. Also required for avascular cartilage development. Defects in HSPG2 are the cause of Schwartz-Jampel syndrome (SJS1); a rare autosomal recessive disorder characterized by permanent myotonia (prolonged failure of muscle relaxation) and skeletal dysplasia, resulting in reduced stature, kyphoscoliosis, bowing of the diaphyses and irregular epiphyses. Defects in HSPG2 are the cause of dyssegmental dysplasia Silverman-Handmaker type (DDSH). The dyssegmental dysplasias are rare, autosomal recessive skeletal dysplasias with anisospondyly and micromelia. There are two recognized types: the severe, lethal DDSH and the milder Rolland-Desbuquois form. Individuals with DDSH also have a flat face, micrognathia, cleft palate and reduced joint mobility, and frequently have an encephalocoele. The endochondral growth plate is short, the calcospherites (which are spherical calcium-phosphorus crystals produced by hypertrophic chondrocytes) are unfused, and there is mucoid degeneration of the resting cartilage.

Protein type: Secreted; Motility/polarity/chemotaxis; Cell adhesion; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1p36.1-p34

Cellular Component: basal lamina; extracellular matrix; extracellular region; extracellular space; focal adhesion; Golgi lumen; lysosomal lumen; plasma membrane

Molecular Function: metal ion binding; protein binding; protein C-terminus binding

Biological Process: angiogenesis; brain development; carbohydrate metabolic process; cardiac muscle development; cellular protein metabolic process; chondrocyte differentiation; chondroitin sulfate metabolic process; embryonic skeletal morphogenesis; endochondral ossification; extracellular matrix disassembly; extracellular matrix organization and biogenesis; fat-soluble vitamin metabolic process; glycosaminoglycan biosynthetic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; lipoprotein metabolic process; phototransduction, visible light; protein localization; retinoid metabolic process; vitamin metabolic process

Disease: Dyssegmental Dysplasia, Silverman-handmaker Type; Schwartz-jampel Syndrome, Type 1